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Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)

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Indolent Lymphomas

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

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Abstract

Extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct lymphoma entity in the WHO-classification and accounts for up to 8% of all B-cell lymphomas. It displays unique clinical and pathological features and is associated with chronic infections including Helicobacter pylori (HP) and Chlamydophila psittaci (CP), as well as autoimmune diseases. MALT lymphoma is an indolent disease as reflected by a 10-year overall survival of 75–80%. In patients with systemic disease, a simple prognostic index (MALT-IPI) including age, stage and LDH has been shown to discriminate between three prognostic risk-groups. Antibiotic therapy is the treatment of choice in gastric MALT lymphoma as well as in some cases of ocular adnexal MALT lymphoma (OAML). HP eradication results in response rates of 80% in gastric MALT lymphoma and 33–65% in OAML. Additionally, radiation and systemic treatment, including anti-CD20 antibodies, as well as chemotherapy and chemo-free approaches are highly effective in the management of these patients.

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Acknowledgements

The authors would like to thank Afua Adjeiwaa Mensah for her critical manuscript review. Support: Supported in part by a grant from Oncosuisse (ICP OCS-02062-03-2007).

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Authors and Affiliations

  1. Division of Medical Oncology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland

    Emanuele Zucca

  2. Institute of Oncology Research, Bellinzona, Switzerland

    Emanuele Zucca

  3. Department of Medical Oncology, University Hospital, Bern, Switzerland

    Emanuele Zucca

  4. Internal Medicine I, Oncology, Medical University of Vienna, Vienna, Austria

    Markus Raderer

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  1. Emanuele Zucca
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Correspondence to Emanuele Zucca .

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  1. Department of Medicine III, LMU Hospital, München, Bayern, Germany

    Martin Dreyling

  2. Azienda Ospedaliera Nazionale SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy

    Marco Ladetto

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Zucca, E., Raderer, M. (2021). Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma). In: Dreyling, M., Ladetto, M. (eds) Indolent Lymphomas . Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-030-55989-2_7

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